Liposarcoma of spermatic cord mimicking an inguinal hernia A case report and review of the literature.

AIM: Liposarcoma of the spermatic cord (LSC) is a tumour often mistaken for common inguinal swelling as hernia and the aim of this work is to present our case with a review of the Literature to define the management of this rare condition. MATERIAL OF STUDY: A systematic review has been realised, considering English language articles published on Pubmed, between 1956 and 2022, using as key words "Liposarcoma of the spermatic cord". RESULTS: 160 studies described 420 cases of LSC and in 40 cases the patient had undergone surgery with an initial diagnosis of inguinal hernia. DISCUSSION: LSC is a very rare entity of genitourinary malignancies, occurring more often in the spermatic cord and diagnosis can be difficult. Our case and Literature data confirm the role of imaging in not conventional inguinal swelling, to avoid diagnostic mistakes and to define preoperatively the correct surgical management. CONCLUSIONS: Imaging is mandatory in case of diagnostic doubt. The recommended treatment is a radical high orchiectomy with clear margins. A long follow-up period is necessary to detect a local recurrence which may occur even several years after the primary therapy. KEY WORDS: Inguinal swelling, Liposarcoma, Spermatic cord.

Decellularized Human Gut as a Natural 3D Platform for Research in Intestinal Fibrosis.

BACKGROUND: The current methodologies for the identification of therapeutic targets for inflammatory bowel disease (IBD) are limited to conventional 2-dimensional (2D) cell cultures and animal models. The use of 3D decellularized human intestinal scaffolds obtained from surgically resected intestine and engineered with human intestinal cells may provide a major advancement in the development of innovative intestinal disease models. The aim of the present study was to design and validate a decellularization protocol for the production of acellular 3D extracellular matrix (ECM) scaffolds from the human duodenum. METHODS: Scaffolds were characterized by verifying the preservation of the ECM protein composition and 3D architecture of the native intestine and were employed for tissue engineering with primary human intestinal myofibroblasts for up to 14 days. RESULTS: Engrafted cells showed the ability to grow and remodel the surrounding ECM. mRNA expression of key genes involved in ECM turnover was significantly different when comparing primary human intestinal myofibroblasts cultured in 3D scaffolds with those cultured in standard 2D cultures on plastic dishes. Moreover, incubation with key profibrogenic growth factors such as TGFß1 and PDGF-BB resulted in markedly different effects in standard 2D vs 3D cultures, further emphasizing the importance of using 3D cell cultures. CONCLUSIONS: These results confirm the feasibility of 3D culture of human intestinal myofibroblasts in intestinal ECM scaffolds as an innovative platform for disease modeling, biomarker discovery, and drug testing in intestinal fibrosis.

Lack of Gut Secretory Immunoglobulin A in Memory B-Cell Dysfunction-Associated Disorders: A Possible Gut-Spleen Axis.

Background: B-1a B cells and gut secretory IgA (SIgA) are absent in asplenic mice. Human immunoglobulin M (IgM) memory B cells, which are functionally equivalent to mouse B-1a B cells, are reduced after splenectomy. Objective: To demonstrate whether IgM memory B cells are necessary for generating IgA-secreting plasma cells in the human gut. Methods: We studied intestinal SIgA in two disorders sharing the IgM memory B cell defect, namely asplenia, and common variable immune deficiency (CVID). Results: Splenectomy was associated with reduced circulating IgM memory B cells and disappearance of intestinal IgA-secreting plasma cells. CVID patients with reduced circulating IgM memory B cells had a reduced frequency of gut IgA+ plasma cells and a disrupted film of SIgA on epithelial cells. Toll-like receptor 9 (TLR9) and transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI) induced IgM memory B cell differentiation into IgA+ plasma cells in vitro. In the human gut, TACI-expressing IgM memory B cells were localized under the epithelial cell layer where the TACI ligand a proliferation inducing ligand (APRIL) was extremely abundant. Conclusions: Circulating IgM memory B cell depletion was associated with a defect of intestinal IgA-secreting plasma cells in asplenia and CVID. The observation that IgM memory B cells have a distinctive role in mucosal protection suggests the existence of a functional gut-spleen axis.

Vaccination coverage and mortality after splenectomy: results from an Italian single-centre study.

Splenectomy is a well-recognised risk factor for life-threatening overwhelming post-splenectomy infection (OPSI). To prevent OPSI, immunisations against encapsulated bacteria (S. pneumoniae, N. meningitidis, H. influenzae) and influenza virus are recommended. However, there is still a lack of uniformity and poor compliance with these recommendations. Following a local physicians' awareness campaign regarding the importance of vaccine prophylaxis of splenectomised patients, we aimed to register vaccination coverage, mortality and infection rates in all patients who underwent splenectomy at our hospital, over a six-year time span. Reasons for splenectomy, patients' compliance with vaccinations, mortality and infectious events were recorded. The reasons for splenectomy in the 216 identified patients (mean age 58.2 ± 14; M:F ratio 1.4:1) were haematologic disorders (38.8%), solid tumours (28.7%), traumatic rupture (22.7%) and other causes (9.7%). A total of 146 patients (67.6%) received at least one of the four vaccines. Overall, the mortality rate was significantly greater in unvaccinated compared to vaccinated patients (p < 0.001), although after the adjustment for the cause of splenectomy the statistical significance was lost (p = 0.085) due to the burden of solid tumour-related mortality. Among the 21 reported cases of OPSI, eight were fatal and five were potentially vaccine-preventable. Our results show that two-thirds of splenectomised patients comply with vaccine prophylaxis. Future interventional studies or ad hoc registries might overcome barriers to vaccination or intentional non-compliance.

Short-term outcomes of single-site robotic cholecystectomy versus four-port laparoscopic cholecystectomy: a prospective, randomized, double-blind trial.

BACKGROUND: Randomized studies could not demonstrate significant outcome benefit after single-incision laparoscopic cholecystectomy compared to classic four-port laparoscopic cholecystectomy (CLC). The new robotic single-site platform might offer potential benefits on local inflammation and postoperative pain due to its technological advantages. This prospective randomized double-blind trial compared the short-term outcomes between single-incision robotic cholecystectomy (SIRC) and CLC. METHODS: Two groups of 30 eligible patients were randomized for SIRC or CLC. During the first postoperative week, patients and study monitors were blinded to the type of procedure performed by four dressing tapes applied on the abdomen. Pain was assessed at 6 h and on day 1, 7 and 30 after surgery, along with a 1-10 cosmetic score. RESULTS: No significant difference in postoperative pain occurred in the two groups at any time point nor for any of the abdominal sites. Nineteen (63 %) SIRC patients reported early postoperative pain in extra-umbilical sites. Intraoperative complications which might influence postoperative pain, such as minor bleeding and bile spillage, were similar in both groups and no conversions occurred. The cosmetic score 1 month postoperatively was higher for SIRC (p < 0.001). Two SIRC patients had wound infection, one of which developed an incisional hernia. CONCLUSIONS: SIRC does not offer any significant reduction of postoperative pain compared to CLC. SIRC patients unaware of their type of operation still report pain in extra-umbilical sites like after CLC. The cosmetic advantage of SIRC should be balanced against an increased risk of incisional hernias and higher costs. TRIAL REGISTRATION NUMBER: ACTRN12614000119695 ( http://www.anzctr.org.au ).

Intra and post-operative complications of esophageal achalasia.

AIM: To evaluate and discuss all the potential complications affecting morbidity of patients treated with surgery for primary achalasia. MATERIAL OF STUDY: A review of the available English literature published to date has been conducted. All articles reporting surgical experience in achalasia were examined and then were selected only those specifically inherent to the topic at issue. RESULTS: Mucosal perforation is the main intra-operative complication while persistence or recurrence of the disease and gastro-esophageal reflux are those mostly affecting patients afterwards, even at long-term follow-up. A few other less common morbidities, as well as the technical considerations useful to minimize and manage each complication mentioned, are reported. DISCUSSION: Minimally invasive surgery for achalasia consent to treat patients with a low rate of perioperative complications that can be managed with conservative approach in the majority of cases. Risk of esophageal cancer exists in these patients and remains although surgical therapy. CONCLUSIONS: Laparoscopic Heller myotomy along with partial fundoplication is a safe and effective procedure that should be considered as the treatment of choice at first evaluation of achalasic patients rather than endoscopic techniques. Robotic technology may add further contribution in diminishing perioperative complications.

Splenic function and IgM-memory B cells in Crohn's disease patients treated with infliximab.

BACKGROUND: Under experimental chronic inflammation, tumor necrosis factor (TNF)-alpha plays a role in damaging spleen marginal zone. This latter has a crucial function in mounting B cell-dependent immune responses against infections by encapsulated bacteria. In Crohn's disease (CD), a chronic inflammatory disorder where TNF-alpha is centrally involved, impaired splenic function may increase the susceptibility to bacterial infections. On this basis, we aimed to investigate the influence of anti-TNF therapy on splenic function in CD patients. METHODS: Peripheral blood samples were obtained from 15 CD patients before and after treatment with infliximab administered at weeks 0, 2, and 6 at a dose of 5 mg/kg. Counting of erythrocytes with membrane abnormalities (pitted red cells) was used as an indicator of splenic function. Multicolor flow cytometry was performed to analyze circulating B cells. RESULTS: A substantial clinical improvement in 10 of the 15 CD patients was associated with a significant reduction of pitted red cells (from median 6.0% to 3.6%; P < 0.01) after 10 weeks of treatment. In responder patients the improvement of splenic function was accompanied by a parallel increase of circulating IgM-memory B cells (from median 6.9% to 13.3%; P < 0.005). Splenic function was not ameliorated in nonresponder patients. CONCLUSIONS: Splenic function improved in CD patients who responded to infliximab and was accompanied by a concomitant restoration of the IgM-memory B cell pool responsible for the protection against encapsulated bacteria. Restoration of splenic function after infliximab treatment is intriguing and requires further investigation.

[Ovarian cyst in a case of female pseudohermaphroditism in a patient with male phenotype]. / Cisti ovarica in paziente a fenotipo maschile e pseudoermafroditismo femminile. Caso clinico.

Female pseudohermaphroditism is an intersexual state distinguished by virilized external genitals and secondary sex characters in a XX subject. We report a case of female pseudohermaphroditism diagnosed later on the discovery of an abdominal mass, then revealed to be an enormous ovarian cyst. Hormonal dosages suggested the presence of partial surrenalic b-hydroxylase deficiency. For this reason the clinical picture was considered expression of an adreno-genital syndrome, displayed as female pseudohermaphroditism with Prader stage V virilization.

[Synchronous and metachronous tumours of colon cancer. A review of 5 years of experience (1999-2004)]. / I tumori sincroni e metacroni del colon-retto. Revisione di 5 anni di attività (1999-2004).

The authors report on 30 synchronous cancer (19%) and 9 metachronous cancer (5.7%) observed in 5 years (1999-2004) in 158 patient operated for colon cancer, defining metachronous cancer a tumor arisen at least 6 months after the first one or further then 5 cm from the anastomosis of the first colon resection. International case records report an incidence of 0.6-14% for synchronous cancer and 1-8% for metachronous cancer. The incidence of synchronous cancer is increasing for the presence of more oncogenic factors in the environment, for the improvement in radiology and endoscopy, for the raise of medium life. In accord with other authors, they show that the main risk factor for the developing of metachronous cancer is the coexistence of colon adenomas at the moment of the diagnosis of the tumor. The various incidence percentages can be explained by different way of diagnosis and classification.

[Different approaches for medical or surgical management of Crohn's disease: the importance of the classification of Vienna]. / Modalità di approccio chirurgico nei pazienti con morbo di Crohn: inquadramento secondo la classificazione di Vienna.

The Vienna classification of Crohn's disease provides defined criteria for a phenotypic classification of the disease, considering that phenotypic parameters reflect the contribution of both genetic and environmental factors to the expression of disease. The classification includes mainly three criteria as part of the natural course of disease: age at the diagnosis, location and behaviour and it provides distinct definitions to categorize Crohn' patients into 24 subgroups. In this study we attempt to define the clinical importance of the Vienna classification and the possibility to optimize medical care of Crohn's disease according to patient subgroups. Early age at the diagnosis is associated with a greater prevalence of a family history, greater small bowel involvement, more complicated stricturing disease and a higher frequency of surgery. Surgery is based on exact knowledge of location: L1 patients might profit the most from resective surgery, L4 patients from strictureplasty. Stricturing behaviour (B2) might be the most appropriate subgroup for surgical treatment. Penetrating behaviour (B3) is confirmed as an important risk for early postoperative recurrence; patients in the penetrating behaviour group (i.e. fistulas) have a specific indication for immunosuppressive or anti-tumour necrosis factor-alpha therapy.

A case of bilateral breast cancer and Graves' disease.

We report a case of bilateral breast cancer associated with Graves' disease characterized by a large goiter and complicated by a severe ophthalmopathy. The hyperthyroidism was treated initially with methimazole and then with thyroidectomy, the ophthalmopathy with intravenous steroids combined with orbital radiotherapy. The breast tumors underwent surgical resection followed by chemotherapy. We describe this case because of the well-known association between breast cancer and thyroid disease, particularly of the autoimmune type, the causes of this being still unclear. Recent literature on this topic is reviewed, discussing the possible role of the Na(+)-I(-) symporter and anti-TPO antibodies.

Doppler enhancement after intravenous levovist injection in Crohn's disease.

Although transabdominal bowel sonography (TABS) has been proposed as a reliable tool to assess increased bowel wall thickness (BWT), the most common sonographic pattern in patients with Crohn's disease (CD), its accuracy is limited in the diagnosis of CD. We therefore tried to assess whether color Doppler enhancement with Levovist, a galactose-based intravenous sonographic contrast agent able to enhance the arterial Doppler signal, increases TABS accuracy. Thirty-one patients with ileal CD, diagnosed by endoscopy and enteroclysis, and 20 healthy volunteers were examined with conventional TABS. Color Doppler of the intramural enteric vessels was then performed before and after intravenous injection of Levovist. Twenty-two CD patients had a BWT >4 mm, and 16 of them presented with active disease. Two of the remaining nine CD patients, all with BWT <4 mm, presented with active disease. By means of color Doppler we identified six patients with inactive disease, normal BWT, and normal basal Doppler signal intensity, who showed an enhanced Doppler signal in intramural vessels after contrast agent bolus. Four of these patients, identified only by color Doppler after Levovist injection, relapsed within 6 months. In our experience, sensitivity and specificity of TABS, integrated with additional stimulated acoustic emission mode, were 96.7% and 100%, respectively. The use of Levovist in color Doppler increases the accuracy of TABS in CD diagnosis and follow-up.